Job's Syndrome clinical trials

Effect of Ranitidine on Hyper-IgE Recurrent Infection (Job's) Syndrome

This study will examine the safety and effectiveness of ranitidine (Zantac) in patients with Hyper-IgE recurrent infection syndrome, a disease characterized by recurrent infections of the ears, sinuses, lungs and skin, and abnormal levels of the antibody immunoglobulin E (IgE). Patients age 2 and older who have Hyper-IgE recurrent infection syndrome and who have had chronic or frequent infections in the last 12 months may be eligible for this study. Participants are randomly assigned to take ranitidine or placebo in pill or liquid form twice a day for 12 months. In addition to treatment, patients undergo the following procedures during visits scheduled on day 0 of the study (baseline) and at 3, 12, 15 and 24 months. Evaluations at 6, 9, 18 and 21 months are by telephone. - Medical history and physical examination - baseline and 3 and 24 months. - Clinical severity score - baseline and 3, 6, 9, 12, 15, 18, 21 and 24 months. - Dermatology exam - baseline and 3, 12, 15 and 24 months. - Pulmonary function test - baseline and 12 and 24 months. - Chest CT - baseline and 12 and 24 months. - Quality of life assessment - baseline and 3, 12, 15 and 24 months. - Pregnancy testing - baseline and 3, 12, 15 and 24 months. - HIV test - baseline and 12 and 24 months. - Contraception evaluation - baseline and 3, 6, 9, 12, 15, 18, 21 and 24 months. - Missed school/work days assessment - baseline and 3, 12, 15 and 24 months. - Medication adherence - baseline and 3, 6, 9, 12, 15, 18, 21 and 24 months. In addition to the above procedures, participants who are not enrolled in study 00-I-0159 have a baseline scoliosis series and genetic consult.